Blood Cells Molecules and Diseases最新文献

{"title":"Sickle cell disease in Indian tribal population: Findings of a multi-centre Indian SCD registry","authors":"Yogita Sharma ,&nbsp;Deepa Bhat ,&nbsp;Parikipandla Sridevi ,&nbsp;Shaily B. Surti ,&nbsp;Manoranjan Ranjit ,&nbsp;Jatin Sarmah ,&nbsp;Godi Sudhakar ,&nbsp;Bontha V. Babu","doi":"10.1016/j.bcmd.2024.102873","DOIUrl":"10.1016/j.bcmd.2024.102873","url":null,"abstract":"<div><h3>Background</h3><p>Sickle cell disease (SCD) registries provide crucial real-world data on demographics, epidemiology, healthcare, patient outcomes, and treatment efficacy. This paper presents findings from the Indian SCD Registry (ISCDR) on clinical manifestations, crisis episodes, disease management, and healthcare utilization in patients with SCD from 12 primary health centres (PHCs) in six tribal districts of India.</p></div><div><h3>Methods</h3><p>The ISCDR was introduced along with a three-tier screening process. Its Android-based application incorporates two electronic case report forms for patient data collection over one year. This paper presents a year's data from the ISCDR's 324 patients with SCD.</p></div><div><h3>Results</h3><p>Patients with SCD, aged one to 65 years, exhibited varied clinical manifestations. Most patients (85.2 %) were unaware of their SCD status before enrolling in ISCDR. Moderate to severe anaemia was prevalent (66.05 % and 30.56 %, respectively). Pain was a common complaint (80.86 %; CI: 76.17–85.00), while symptoms of stroke included sudden severe headaches (34.57 %; CI: 29.40–40.02). Common splenic sequestration symptoms included stomach pain (42.90 %; CI: 37.44–48.49) and abdominal tenderness (13.27 %; CI: 9.77–17.46), as a sign. Healthcare utilization was high, with 96.30 % receiving treatment and 83.64 % consuming hydroxyurea. Hospitalization occurred for 38.27 % (CI: 32.95–43.81), and 12.04 % (CI: 8.70–16.09) had blood transfusion during last year.</p></div><div><h3>Conclusions</h3><p>ISCDR serves as a dynamic digital database on SCD epidemiology, clinical aspects, treatment and healthcare utilization. Notably, many patients lacked prior awareness of their SCD status, underscoring the need for improved awareness and care management. Integrating the registry into the national programme can streamline treatment implementation, prioritize management approaches, and optimize individual benefits.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102873"},"PeriodicalIF":2.1,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141637396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Accelerated phase development in a late-onset adolescent Chediak-Higashi syndrome patient caused by compound novel LYST mutations in the setting of SARS-CoV-2 infection","authors":"Ping Guo ,&nbsp;Xi Wu ,&nbsp;Mingkang Yang,&nbsp;Yilun Xue,&nbsp;Jiakuan Zhou,&nbsp;Zhixi Huang,&nbsp;Wenman Wu,&nbsp;Jianbiao Wang","doi":"10.1016/j.bcmd.2024.102874","DOIUrl":"10.1016/j.bcmd.2024.102874","url":null,"abstract":"<div><p>Chediak-Higashi syndrome (CHS) is a rare autosomal recessive genetic disorder characterized by severe immunodeficiency, albinism and coagulation deficiency. Mostly diagnosed in early childhood, this devastating condition is associated with lysosomal abnormalities attributed to the absence or impaired function of lysosomal trafficking regulator caused by mutations in the <em>CHS1/LYST</em> gene. In current study, we report a case of late-onset CHS caused by two novel compound heterozygous <em>CHS1/LYST</em> mutations: c.8407C &gt; T, leading to early termination of translation at residue Gln2803 (p. Gln2803Ter), and a small deletion c. 4020_4031del, resulting in an in-frame deletion of three amino acid residues (p. Asp1343_Val1346del). Both variants retain a large part of the CHS/LYST protein, particularly p. Asp1343_Val1346del, which preserves critical functional BEACH and WD40 domains in the C terminal, potentially maintaining residual activity and alleviating patient symptoms. The timeline of SARS-CoV-2 infection and rapid symptom progression suggests that the viral infection may have trigger the accelerated phase development leading to a poor prognosis.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102874"},"PeriodicalIF":2.1,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141698963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molecular and biochemical evaluation of oxidative effects of cord blood CD34+ MPs on hematopoietic cells","authors":"Zoi Katana ,&nbsp;Kyriaki Sianidou ,&nbsp;Gregory Kaiopoulos ,&nbsp;Fani Deligianni ,&nbsp;Sarantis Tsetsakos ,&nbsp;Anastasia Kouvatsi ,&nbsp;Ioanna Sakellari ,&nbsp;Aristeidis Kritis ,&nbsp;Maria Touraki ,&nbsp;Damianos Sotiropoulos ,&nbsp;Angeliki Xagorari","doi":"10.1016/j.bcmd.2024.102871","DOIUrl":"10.1016/j.bcmd.2024.102871","url":null,"abstract":"<div><p>A graft source for allogeneic hematopoietic stem cell transplantation is umbilical cord blood, which contains umbilical cord blood mononuclear cells (MNCs and mesenchymal stem cells, both an excellent source of extracellular microparticles (MPs). MPs act as cell communication mediators, which are implicated in reactive oxygen species formation or detoxification depending on their origin. Oxidative stress plays a crucial role in both the development of cancer and its treatment by triggering apoptotic mechanisms, in which CD34+ cells are implicated. The aim of this work is to investigate the oxidative stress status and the apoptosis of HL-60 and mononuclear cells isolated from umbilical cord blood (UCB) following a 24- and 48-hour exposure to CD34 + microparticles (CD34 + MPs). The activity of superoxide dismutase, glutathione reductase, and glutathione S-transferase, as well as lipid peroxidation in the cells, were employed as oxidative stress markers. A 24- and 48-hour exposure of leukemic and mononuclear cells to CD34 + -MPs resulted in a statistically significant increase in the antioxidant activity and lipid peroxidation in both cells types. Moreover, CD34 + MPs affect the expression of BCL2 and FAS and related proteins and downregulate the hematopoietic differentiation program in both HL-60 and mononuclear cells. Our results indicate that MPs through activation of antioxidant enzymes in both homozygous and nonhomozygous cells might serve as a means for graft optimization and enhancement.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"108 ","pages":"Article 102871"},"PeriodicalIF":2.1,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141623690","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dysmorphic megakaryocytes in TAFRO syndrome: A case series from a single institute","authors":"Shohei Maida ,&nbsp;Hiromi Nakagawa ,&nbsp;Hiroshi Ureshino ,&nbsp;Kyoko Kajihara ,&nbsp;Shinichi Yamazaki ,&nbsp;Tatsuo Ichinohe","doi":"10.1016/j.bcmd.2024.102870","DOIUrl":"10.1016/j.bcmd.2024.102870","url":null,"abstract":"<div><p>TAFRO syndrome is a rare systemic inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. The diagnosis of TAFRO syndrome can be challenging; however, prompt diagnosis is vital because TAFRO syndrome is a progressive and life-threatening disease. We have showcased five patients with TAFRO syndrome who had similar bone marrow (BM) findings that could be considered the findings that characterize TAFRO syndrome. All patients were treated with corticosteroids and tocilizumab; three of the five patients (60 %) responded positively to the treatment. The unique BM findings observed in this study were megakaryocytes with distinct multinuclei and three-dimensional and indistinct bizarre nuclei (“dysmorphic megakaryocyte”), similar to the megakaryocyte morphology observed in myeloproliferative neoplasms (MPNs). Notably, dysmorphic megakaryocytes were observed in all five cases, whereas only two of the five patients tested positive for reticulin myelofibrosis, and three of the five patients had megakaryocytic hyperplasia, which are considered typical findings of TAFRO syndrome. Thus, the BM findings of dysmorphic megakaryocytes could help in the correct and immediate diagnosis of TAFRO syndrome.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"108 ","pages":"Article 102870"},"PeriodicalIF":2.3,"publicationDate":"2024-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141404724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical procedures and complications in placement of totally implantable venous access port in pediatric hemophilia patients: A retrospective analysis","authors":"Wei Cheng ,&nbsp;Jinrui Zhang ,&nbsp;Xipeng Wang ,&nbsp;Guoqing Liu ,&nbsp;Wanru Yao ,&nbsp;Chunli Wang ,&nbsp;Runhui Wu ,&nbsp;Zhiqiang Li","doi":"10.1016/j.bcmd.2024.102862","DOIUrl":"10.1016/j.bcmd.2024.102862","url":null,"abstract":"<div><p>This retrospective study at Beijing Children's Hospital (2020–2023) analyzed surgical procedures and complications in 24 pediatric hemophilia patients undergoing Totally Implantable Venous Access Port (TIVAP) insertion, primarily in the right jugular vein (RJV). We detailed the surgical process, including patient demographics and intraoperative imaging use. The choice of the RJV for TIVAP placement was influenced by its larger diameter and superficial anatomical position, potentially reducing risks like thrombosis and infection. Our findings support the RJV as a safer alternative for port placement in pediatric patients, aligning with current literature. Statistical analysis revealed no significant correlation between complications and baseline characteristics like weight and diagnosis type. However, the length of hospital stay and implant brand were significant risk factors for catheter or port displacement and removal. The limited patient number may introduce bias, suggesting a need for further studies with larger samples. Despite a 14.7 %–33 % complication rate and 5 port removals, the advantages of TIVAP, including reliable venous access, reduced discomfort, and treatment convenience, were evident. Most complications improved with symptomatic treatment, and there were no deaths due to port-related complications, underscoring the impact of TIVAP on improving pediatric hemophilia treatment.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"108 ","pages":"Article 102862"},"PeriodicalIF":2.3,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1079979624000408/pdfft?md5=2b35836cd4ee944b58be12582d724ec4&pid=1-s2.0-S1079979624000408-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141403393","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mechanism of the apoptosis of bone marrow erythroblasts in rats under hypobaric hypoxia","authors":"","doi":"10.1016/j.bcmd.2024.102861","DOIUrl":"10.1016/j.bcmd.2024.102861","url":null,"abstract":"<div><p>This study aimed to investigate the mechanism of the apoptosis of erythroblasts in rat bone marrow after the exposure to hypobaric hypoxia. Male SD rats were randomly divided into three groups. The hypoxic group was kept in a hypobaric hypoxia chamber at a simulated altitude of 5000 m for 7 and 28 days, respectively. The control group was kept at an altitude of 2260 m. We found that myeloid: erythroid (M:E) ratio was significantly lower after hypoxia exposure and the proportions of polychromatic erythroblasts and orthochromatic erythroblasts significantly increased compared to control group, along with significant increase in the proportion of CD71+ cells and apoptosis rate. The expression levels of caspase-3, Bax, and Cyt-C in CD71+ cells were higher after hypoxia exposure than those in control group, while there was no significant difference in the expression levels of TNFR and Fas. In conclusion, after exposure to hypobaric hypoxia the proliferation of peripheral blood and bone marrow erythroblasts in rats increased, and apoptosis also increased, indicating that bone marrow erythroblasts in rats is regulated by both proliferation and apoptosis, and the mitochondrial pathway is one of the important pathways for apoptosis.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"108 ","pages":"Article 102861"},"PeriodicalIF":2.1,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Differential effects of iron chelators on iron burden and long-term morbidity and mortality outcomes in a large cohort of transfusion-dependent β-thalassemia patients who remained on the same monotherapy over 10 years","authors":"Khaled M. Musallam ,&nbsp;Susanna Barella ,&nbsp;Raffaella Origa ,&nbsp;Giovanni Battista Ferrero ,&nbsp;Roberto Lisi ,&nbsp;Annamaria Pasanisi ,&nbsp;Filomena Longo ,&nbsp;Barbara Gianesin ,&nbsp;Gian Luca Forni ,&nbsp;Webthal® project","doi":"10.1016/j.bcmd.2024.102859","DOIUrl":"10.1016/j.bcmd.2024.102859","url":null,"abstract":"<div><p>We conducted a retrospective cohort study on 663 transfusion-dependent β-thalassemia patients receiving the same iron chelation monotherapy with deferoxamine, deferiprone, or deferasirox for up to 10 years (median age 31.8 years, 49.9 % females). Patients on all three iron chelators had a steady and significant decline in serum ferritin over the 10 years (median deferoxamine: −170.7 ng/mL, <em>P</em> = 0.049, deferiprone: −236.7 ng/mL, <em>P</em> = 0.001; deferasirox: −323.7 ng/mL, <em>P</em> &lt; 0.001) yet had no significant change in liver iron concentration or cardiac T2*; while noting that patients generally had low hepatic and cardiac iron levels at study start. Median absolute, relative, and normalized changes were generally comparable between the three iron chelators. Patients receiving deferasirox had the highest morbidity and mortality-free survival probability among the three chelators, although the difference was only statistically significant when compared with deferoxamine (<em>P</em> = 0.037). On multivariate Cox regression analysis, there was no significant association between iron chelator type and the composite outcome of morbidity or mortality. In a real-world setting, there is comparable long-term iron chelation effectiveness between the three available iron chelators for patients with mild-to-moderate iron overload.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"107 ","pages":"Article 102859"},"PeriodicalIF":2.3,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141183152","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP)","authors":"A. Guarina ,&nbsp;P. Farruggia ,&nbsp;E. Mariani ,&nbsp;P. Saracco ,&nbsp;A. Barone ,&nbsp;D. Onofrillo ,&nbsp;S. Cesaro ,&nbsp;R. Angarano ,&nbsp;W. Barberi ,&nbsp;S. Bonanomi ,&nbsp;P. Corti ,&nbsp;B. Crescenzi ,&nbsp;G. Dell'Orso ,&nbsp;A. De Matteo ,&nbsp;G. Giagnuolo ,&nbsp;A.P. Iori ,&nbsp;S. Ladogana ,&nbsp;A. Lucarelli ,&nbsp;M. Lupia ,&nbsp;B. Martire ,&nbsp;C. Dufour","doi":"10.1016/j.bcmd.2024.102860","DOIUrl":"10.1016/j.bcmd.2024.102860","url":null,"abstract":"<div><p>Acquired aplastic anemia (AA) is a rare heterogeneous disorder characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2–3 per million population per year in the Western world, but 3 times higher in East Asia. Survival in severe aplastic anemia (SAA) has improved significantly due to advances in hematopoietic stem cell transplantation (HSCT), immunosuppressive therapy, biologic agents, and supportive care. In SAA, HSCT from a matched sibling donor (MSD) is the first-line treatment. If a MSD is not available, options include immunosuppressive therapy (IST), matched unrelated donor, or haploidentical HSCT. The purpose of this guideline is to provide health care professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary evidence-based document prepared by a group of pediatric hematologists of the Bone Marrow Failure Study Group of the Italian Association of Pediatric Hemato-Oncology (AIEOP) was discussed, modified and approved during a series of consensus conferences that started online during COVID 19 and continued in the following years, according to procedures previously validated by the AIEOP Board of Directors.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"108 ","pages":"Article 102860"},"PeriodicalIF":2.3,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141417573","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aberrant baseline cytokine profile in patients with newly diagnosed acquired aplastic anaemia correlates with disease severity and the treatment response","authors":"Rahul Vatsayan ,&nbsp;Ankur Jain ,&nbsp;Aditya Jandial ,&nbsp;Parveen Bose ,&nbsp;Man Updesh Singh Sachdeva ,&nbsp;Neelam Varma ,&nbsp;Arihant Jain ,&nbsp;Gaurav Prakash ,&nbsp;Alka Khadwal ,&nbsp;Pankaj Malhotra","doi":"10.1016/j.bcmd.2024.102857","DOIUrl":"10.1016/j.bcmd.2024.102857","url":null,"abstract":"<div><h3>Background</h3><p>Immune dysregulation is crucial in the pathogenesis of acquired aplastic anaemia (aAA). There is paucity of data regarding correlation of baseline cytokine profile with treatment response in aAA.</p></div><div><h3>Objective</h3><p>Present prospective case-control study aimed to correlate the baseline cytokines in patients with aAA with the treatment response.</p></div><div><h3>Methods</h3><p>Fifty-one patients with newly-diagnosed aAA &gt; 13 years of either sex were enrolled over 1.5 years. Twenty age-and sex-matched healthy controls (HC) were also included. The cytokine profile (IL-2, 4, 6, 8, 10, 17, IFN-γ and TNF-α) in the peripheral blood plasma of aAA patients was performed at the baseline using cytometric bead analysis. The cytokine levels were compared with HC and correlated with response to immunosuppressive therapy (IST) at 3-months.</p></div><div><h3>Results</h3><p>The median age of cases was 29 years (range,13–74). The cases had higher mean levels of IL2 (<em>p</em> = 0.326), IL4 (<em>p</em> = 0.038), IL6 (<em>p</em> = 0.000), IL10 (<em>p</em> = 0.002), TNF-α (<em>p</em> = 0.302), IFN-γ (<em>p</em> = 0.569) and IL-17 (<em>p</em> = 0.284) than the HC. The baseline levels of all the cytokines were higher (statistically non-significant) among responders (<em>n</em> = 13) than the non-responders (<em>n</em> = 14) to IST.</p></div><div><h3>Conclusions</h3><p>Baseline cytokine profile in patients with aAA might predict response to the IST. Larger studies are needed to validate our results.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"107 ","pages":"Article 102857"},"PeriodicalIF":2.3,"publicationDate":"2024-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141030757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Daucosterol regulates JAK2-STAT3 signaling pathway to promote megakaryocyte differentiation","authors":"Zhongkang Zhang,&nbsp;Guangbin Shang,&nbsp;Zhen Lu,&nbsp;Jia Hu,&nbsp;Huizhen Liu,&nbsp;Ting Lu,&nbsp;Xiaonan Lu","doi":"10.1016/j.bcmd.2024.102858","DOIUrl":"10.1016/j.bcmd.2024.102858","url":null,"abstract":"<div><p>Immune thrombocytopenia (ITP) is an autoimmune disease caused by the loss of immune tolerance to platelet autoantigens, resulting in reduced platelet production and increased platelet destruction. Impaired megakaryocyte differentiation and maturation is a key factor in the pathogenesis and treatment of ITP. <em>Sarcandra glabra</em>, a plant of the Chloranthaceae family, is commonly used in clinical practice to treat ITP, and daucosterol (Dau) is one of its active ingredients. However, whether Dau can treat ITP and the key mechanism of its effect are still unclear. In this study, we found that Dau could effectively promote the differentiation and maturation of megakaryocytes and the formation of polyploidy in the megakaryocyte differentiation disorder model constructed by co-culturing Dami and HS-5 cells. In vivo experiments showed that Dau could not only increase the number of polyploidized megakaryocytes in the ITP rat model, but also promote the recovery of platelet count. In addition, through network pharmacology analysis, we speculated that the JAK2-STAT3 signaling pathway might be involved in the process of Dau promoting megakaryocyte differentiation. Western blot results showed that Dau inhibited the expression of P-JAK2 and P-STAT3. In summary, these results provide a basis for further studying the pharmacological mechanism of Dau in treating ITP.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"107 ","pages":"Article 102858"},"PeriodicalIF":2.3,"publicationDate":"2024-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141032116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}